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Table 3 The percentage of respondents that endorsed problem behaviors

From: Neurobehavioral and developmental profiles: genotype–phenotype correlations in individuals with Cornelia de Lange syndrome

 

N (sample size)

NIPBL

N (sample size)

SMC1A

p Value

Never a problem (%)

Not a problem today, but was in the past (%)

Currently a problem (%)

Never a problem (%)

Not a problem today, but was in the past (%)

Currently a problem (%)

Presents with temper tantrums

23

30.4

0

69.6

11

72.7

0

27.3

FET p = 0.059

Difficult to reach or get through

20

35

25

40

9

55.6

44.4

0

FET p = 0.079

Isolates self

23

34.8

26.1

39.1

11

81.8

18.2

0

FET p = 0.015

Attention

 Excessively active

22

68.2

4.5

27.3

10

30

30

40

FET p = 0.063

 Restless

22

45.5

4.5

50

10

30

20

50

n.s

Does not pay attention to instructions

21

14.3

9.5

76.2

10

20

20

60

n.s

 Easily distractible

21

14.3

14.3

71.4

11

18.2

9.1

72.7

n.s

Repetitive/unusual behaviors

 Rocks body back and forth

24

87.5

0

12.5

11

90.9

0

9.1

n.s

 Spins, twirls, paces

24

25

33.3

41.7

11

81.8

18.2

0

FET p = 0.004

 Need to line up items or make symmetrical

24

45.8

16.7

37.5

11

81.8

0

18.2

n.s

 Unable to throw things away

22

81.8

9.1

9.1

11

90.9

9.1

0

n.s

  1. FET Fisher’s exact test, n.s. not significant (p > 0.10)